POS0069 LONGITUDINAL CHANGES ON QUANTITATIVE CHEST HIGH-RESOLUTION COMPUTED TOMOGRAPHY IN EARLY DIFFUSE SYSTEMIC SCLEROSIS-RELATED INTERSTITIAL LUNG DISEASE AFTER AUTOLOGOUS HEMATOPOIETIC STEM CELL TRANSPLANTATION.
Authors: G. Pugnet, A. Petermann, S. Collot, P. Otal, P. Lansiaux, A. Maria, N. Ait Abdallah, G. Lorillon, M. Resch-Rigon, Z. Marjanovic, D. Farge
Journal: Annals of the Rheumatic Diseases (2022)

<h3>Background</h3> Since Burt <i>et al.</i> first demonstrated in the ASSIST trial (1) that lung disease high-resolution computed tomography (HRCT) volumetric measurement decreased in autologous hematopoietic stem cell transplantation (aHSCT) recipients and increased in controls receiving cyclophosphamide iv one year after starting treatment, limited data have been reported concerning the evolution of Systemic Sclerosis-Interstitial Lund disease (SSc-ILD) since and its relationship with the evolution of esophageal volume is unknown. <h3>Objectives</h3> To evaluate High-Resolution Computed Computed Tomography (HRCT) interstitial lung disease (ILD) and esophageal involvement in early diffuse systemic sclerosis (dSSc)-patients before and after autologous Hematopoietic Stem Cell Transplantation (aHSCT). <h3>Methods</h3> Overall chest HRCT, lung function and skin score changes were evaluated in 33 consecutive dSSc-patients before and after aHSCT during yearly routine follow-up between January 2000 and september 2016.Two independent radiologists blindly assessed the ILD extent using semi-quantitative Goh and Wells method (2), the widest esophageal diameter (WED) and the esophageal volume (EV) on HRCT. Patients were retrospectively classified as radiological responders or non-responders at 24 months after aHSCT according to the stability or a 5% or more decrease of HRCT ILD extent. Two by two time points comparisons after versus before aHSCT were performed using linear regressions. Survival rates were assessed for all patients by Kaplan-Meier method. <h3>Results</h3> Twenty-four months after aHSCT, HRCT median [IQR] ILD (-2 [-10.3; 0] points, p=0.0002) and ground-grass opacities (-2.1 [-8.3; 0] points, p=0.02) extent scores had improved, with 18 patients radiological responders (probability of response 0.78, 95% CI (0.58;0.90), whereas median WED (from 24.5 [18; 29] to 28 [19; 33] mm; p= 0.005) and EV (from 19 [13; 33] to 30 [13; 58] mm<sup>3</sup>, p=0.01) increased significantly. Kaplan-Meier analyses showed a trend towards better 5 years survival rates (100% versus 60%; HR 0.23 95%CI 0.03 to 1.62, P = 0.11) among radiological responders versus non-responders at 24 months. <h3>Conclusion</h3> Real-world data analysis confirmed significant HRCT SSc-ILD improvement 24 months after aHSCT, although esophageal dilatation worsened requiring specific attention. <h3>References</h3> [1]Burt RK, Shah SJ, Dill K, Grant T, Gheorghiade M, Schroeder J, et al. Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomised phase 2 trial. Lancet Lond Engl. 6 août 2011;378(9790):498‑506. [2]Goh NSL, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 1 juin 2008;177(11):1248‑54. <h3>Disclosure of Interests</h3> None declared